Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate (rarely metastasizing) biologic potential and uncertain differentiation, which predominantly arises superficially in the deep dermis and subcutis of the extremities of children and young adults.
Angiomatoid fibrous histiocytoma is rare, accounting for approximately only 0.3% of all soft tissue tumors, (4) although given its histologic similarity to a variety of other neoplasms it is likely that it has been previously underdiagnosed and subsumed under a variety of other neoplastic categories, including vascular, fibrohistiocytic, and myofibroblastic types.
Angiomatoid fibrous histiocytoma does not have a proven relationship with any other specific conditions, although one case has been reported on the knee of a child with human immunodeficiency virus.
Angiomatoid fibrous histiocytoma shows a wide morphologic spectrum, and the only constant finding is of sheets and short fascicles of ovoid, epithelioid, or spindle cells with bland, vesicular nuclei (Figure 1).
Angiomatoid fibrous histiocytoma lacks a specific immunoprofile, so that immunohistochemistry is supportive rather than diagnostic.
Angiomatoid fibrous histiocytoma is associated with the following 3 characteristic translocations" t(2:22)(q33:q12) (forming the EWSR1-CREB1 fusion gene), (43,44) t(12:22)(q13:q12) (forming the EWSR1-ATF1 fusion gene) (7,44-46) and t(12:16)(q13:p11) (resulting in the FUS-ATF1 fusion gene).
Angiomatoid fibrous histiocytoma is a rare neoplasm of intermediate biologic potential most frequently occurring in the superficial extremities of children and young adults.
Angiomatoid fibrous histiocytoma. In: Fletcher CDM, Unii KK, Mertens F eds.
Primary intracerebral angiomatoid fibrous histiocytoma" report of a case with a t(12; 22)(q13; q12) causing type 1 fusion of the EWS and ATF-1 genes.
Intracranial angiomatoid fibrous histiocytoma presenting as recurrent multifocal intraparenchymal hemorrhage.
Angiomatoid fibrous histiocytoma of bone" a calcifying sclerosing variant mimicking osteosarcoma.